Tricuspid stenosis

INTRODUCTION — Tricuspid stenosis (TS) is an uncommon valvular abnormality that is usually found in combination with tricuspid regurgitation and other valvular lesions. TS is rarely an isolated valve lesion.

The clinical features of TS and the management of patients with this lesion will be reviewed here.

The diagnosis and management of tricuspid regurgitation is discussed separately. (See "Etiology, clinical features, and evaluation of tricuspid regurgitation" and "Management and prognosis of tricuspid regurgitation".)

Diagnosis and management of tricuspid prosthetic valve obstruction are discussed separately. (See "Bioprosthetic valve thrombosis, thromboembolism, and obstruction: Clinical manifestations and diagnosis" and "Bioprosthetic valve thrombosis, thromboembolism, and obstruction: Management" and "Mechanical prosthetic valve thrombosis or obstruction: Clinical manifestations and diagnosis" and "Management of mechanical prosthetic valve thrombosis and obstruction".)

ETIOLOGY — Causes of native TS include the following conditions [1,2]:

●Acquired causes:

•Rheumatic heart disease is the most common cause of TS. Most cases of tricuspid rheumatic disease present with tricuspid regurgitation or a combination of regurgitation and stenosis; pure TS is very uncommon [3,4]. Rheumatic tricuspid disease almost never occurs as an isolated lesion but is nearly always associated with mitral valve disease and, in some cases, aortic valve disease [5,6]. In an angiographic study of 525 patients with rheumatic heart disease, 9 percent had TS, and all patients with TS also had significant tricuspid regurgitation [7].

•Carcinoid syndrome is a cause of TS [8], although tricuspid regurgitation is more common and is generally predominant when combined with TS. (See "Clinical features of carcinoid syndrome".)

•Benign or malignant tumors in the right heart can obstruct flow through the tricuspid valve and thus cause functional TS.

•Hypereosinophilic syndromes rarely cause TS [9]. Hypereosinophilia has been linked to endomyocardial fibrosis which is commonly associated with mitral or tricuspid regurgitation and rarely associated with TS [10]. (See "Endomyocardial fibrosis" and "Hypereosinophilic syndromes: Clinical manifestations, pathophysiology, and diagnosis".)

•Infective endocarditis rarely causes isolated TS but may cause mixed tricuspid disease (tricuspid regurgitation and TS), particularly when large vegetations are present [11]. (See "Right-sided native valve infective endocarditis".)

•Nonbacterial thrombotic endocarditis (NBTE, also known as Libman-Sacks endocarditis or marantic endocarditis) is a form of noninfectious endocarditis with sterile platelet thrombi deposition on heart valves (predominantly aortic and mitral, with the tricuspid valve less commonly involved [12]). NBTE is associated with a number of conditions including advanced malignancy, systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, sepsis, and burns. (See "Nonbacterial thrombotic endocarditis" and "Clinical manifestations and diagnosis of systemic lupus erythematosus in adults", section on 'Clinical manifestations'.)

•Fabry disease is commonly associated with mitral, tricuspid, or aortic regurgitation; it is rarely associated with TS [13]. (See "Fabry disease: Cardiovascular disease".)

•Light chain amyloid deposition has been documented to cause concomitant moderately severe tricuspid and mitral valve stenosis [14].

•Iatrogenic causes:

-Injury of the tricuspid valve apparatus associated with endocardial pacemaker lead(s) or endomyocardial biopsy procedures (with [15-18] or without [19,20] associated infection [21]). Rarely, large tricuspid vegetations may cause stenosis. Pacemaker lead looping may also interfere with valve opening. (See "Infections involving cardiac implantable electronic devices: Epidemiology, microbiology, clinical manifestations, and diagnosis".)

-Radiation therapy.

-Ergotamine therapy. This is a rare cause of TS [22]. (See "Valvular heart disease induced by drugs", section on 'Ergot derivatives'.)

-A malaligned ventricular septal occluder device. This is a rare cause of TS with tricuspid regurgitation [23].

●Congenital causes include congenital tricuspid valve atresia or stenosis of the valve including an atypical presentation of Ebstein anomaly [24,25] (which is usually associated with tricuspid regurgitation rather than TS). (See "Tricuspid valve atresia" and "Ebstein anomaly: Clinical manifestations and diagnosis".)

Prosthetic tricuspid valve stenosis is an increasingly common lesion and may occur with [26] or without associated endocarditis [27,28]. (See "Bioprosthetic valve thrombosis, thromboembolism, and obstruction: Clinical manifestations and diagnosis".)

PATHOPHYSIOLOGY — As a result of tricuspid valvular stenosis, there is a persistent diastolic pressure gradient between the right atrium and right ventricle. This gradient increases when blood flow across the tricuspid valve increases, as occurs with inspiration and exercise, and decreases when blood flow decreases, such as with expiration. (See "Hemodynamics of valvular disorders as measured by cardiac catheterization".)

As a result, most patients with significant TS have elevated right atrial pressures and display clinical features suggestive of systemic venous congestion, such as jugular venous distension, ascites, and peripheral edema. (See "Cardiac catheterization techniques: Normal hemodynamics" and "Examination of the jugular venous pulse".)

During sinus rhythm, the right atrial a-wave is increased and may approach the level of right ventricular systolic pressure. When severe TS is present, the resting cardiac output may be reduced and fails to increase with exercise. In patients with significant mitral valve disease and significant TS, the left atrial and pulmonary pressures may be only modestly elevated due to proximal coexisting severe TS.

Reverse Lutembacher syndrome is a rare cause of hypoxia characterized by the triad of TS, elevated right atrial pressure, and an interatrial right-to-left shunt [29].

CLINICAL MANIFESTATIONS — The clinical features of isolated TS arise from a combination of obstruction to tricuspid flow, which limits cardiac output and may cause fatigue, and elevated right atrial pressure, which produces symptoms and signs of systemic venous hypertension. When TS is not an isolated lesion, the clinical features of systemic congestion are usually detected, but the associated clinical features of coexisting TR or left-sided valve lesions may be more prominent, and, consequently, TS is often not suspected. (See "Clinical manifestations and diagnosis of rheumatic heart disease", section on 'Chronic valve disease' and "Rheumatic mitral stenosis: Clinical manifestations and diagnosis".)

Symptoms — In the absence of mitral or aortic valve disease, symptoms and signs of systemic venous hypertension are generally out of proportion to dyspnea. However, most patients with TS (particularly those with rheumatic heart disease) have significant mitral and/or aortic valve disease [4,5]. (See "Clinical manifestations and diagnosis of rheumatic heart disease".)

Patients with TS often complain of abdominal discomfort, which is due to the presence of hepatomegaly secondary to hepatic congestion. Fatigue and effort intolerance may be caused by diminished cardiac output. Some patients may sense a fluttering discomfort in the neck, caused by tall a-waves in the jugular venous pulse. (See 'Physical examination' below.)

Physical examination — Physical findings include jugular venous distension, hepatomegaly and hepatic pulsations, ascites, peripheral edema, and, occasionally, anasarca. The findings seen on physical examination in patients with TS are similar to those of mitral stenosis. Since these two lesions often coexist, the diagnosis of TS may be missed.

Obstruction to flow across the tricuspid valve produces an increase in right atrial and jugular venous pressure. Jugular venous pulsations often exhibit a prominent presystolic a-wave, which may be confused with an arterial pulsation. The specificity of a prominent a-wave is reduced when coexisting pulmonary hypertension is present. The y-descent is slow and barely appreciated. Kussmaul sign (lack of decrease or a rise in jugular venous pressure with inspiration) may be seen. A jugular or clavicular presystolic click may be heard [30]. (See "Examination of the jugular venous pulse".)

The lungs are clear in patients with isolated TS. Signs of pulmonary congestion are present in some patients with concurrent mitral valve disease. (See "Clinical manifestations and diagnosis of chronic mitral regurgitation", section on 'Physical examination' and "Rheumatic mitral stenosis: Clinical manifestations and diagnosis", section on 'Physical examination'.)

On auscultation, an opening snap of the tricuspid valve has been described but, when mitral stenosis is also present, it would be difficult to distinguish from the opening snap of mitral stenosis. The tricuspid opening snap usually follows the opening snap of the mitral valve and is localized to the lower left sternal border. (See "Auscultation of heart sounds", section on 'Tricuspid valve'.)

A low frequency diastolic murmur is heard at the lower left sternal border in the fourth intercostal space; it is usually softer, higher pitched, and shorter in duration than the murmur of mitral stenosis. The intensity of the murmur and opening snap in TS increases with maneuvers that increase blood flow across the tricuspid valve, especially with inspiration (Carvallo sign), and also with leg raising, inhalation of amyl nitrate, squatting, or exercise. A middiastolic murmur in the tricuspid valve area may also occur in the absence of TS in patients with a large atrial septal defect, but an opening snap will not be present. (See "Auscultation of cardiac murmurs in adults", section on 'Tricuspid stenosis' and "Physiologic and pharmacologic maneuvers in the differential diagnosis of heart murmurs and sounds".)

Laboratory tests — Liver biochemical test abnormalities, including mild elevation in the serum bilirubin (most of which is unconjugated), occur in patients with hepatic congestion. Other liver biochemical tests, such as serum alkaline phosphatase and serum aminotransferase levels, may be normal or mildly increased. Serum albumin levels may be normal or mildly depressed. (See "Congestive hepatopathy".)

Chest radiograph — A chest radiograph is not indicated to diagnose TS but may be obtained in patients with dyspnea caused by concomitant valve disease (eg, mitral stenosis). The nonspecific finding of right atrial enlargement is seen with TS. In patients with concomitant mitral valve disease, additional findings may be seen, such as evidence of pulmonary edema (including redistribution of pulmonary blood flow to the upper lobes, and interstitial edema), left atrial enlargement, or a straight left heart border. (See "Rheumatic mitral stenosis: Clinical manifestations and diagnosis", section on 'Chest radiograph'.)

In patients with TS caused by endocardial pacemaker lead(s), looped atrial or ventricular leads are commonly (but not always) seen on the chest radiograph [19].

Electrocardiogram — An electrocardiogram is not indicated to diagnose TS but is commonly obtained in the evaluation of patients with valvular heart disease to identify concurrent conditions such as atrial fibrillation (which occurs frequently in patients with rheumatic heart disease) and as a baseline for future comparison. In patients with TS in sinus rhythm, the electrocardiogram may show tall, peaked P waves in leads II, III, and avF consistent with right atrial enlargement [31]. Most patients with rheumatic heart disease have concurrent mitral disease, with associated electrocardiographic changes, including signs of left atrial enlargement and atrial fibrillation. (See "Rheumatic mitral stenosis: Clinical manifestations and diagnosis", section on 'Electrocardiogram'.)

DIAGNOSIS AND EVALUATION

Approach to diagnosis and evaluation — TS should be suspected in patients with signs and symptoms of systemic venous hypertension, particularly those with conditions associated with TS. TS related to pacemaker leads should be suspected in patients with chronic endocardial leads presenting with right heart failure. (See 'Etiology' above.)

TS is generally diagnosed and evaluated by transthoracic echocardiogram, which is often performed to evaluate other associated suspected valve lesions.

Cardiac catheterization for hemodynamic assessment of the severity of TS is generally not required but is suggested in symptomatic patients when clinical and noninvasive data are discordant. When patients with left-sided valve disease and pulmonary hypertension undergo catheterization, an assessment for TS should be performed.

Echocardiography — The diagnosis of TS is generally based upon identifying the following combination of echocardiographic abnormalities [32]. The following echocardiographic features are seen with TS:

●Tricuspid valve leaflets are thickened and distorted with limited leaflet mobility, reduced separation of the leaflet tips, and diastolic doming of the valve. The degree of thickening and calcification with rheumatic TS is generally less pronounced than that seen in rheumatic mitral stenosis. (See "Echocardiographic evaluation of the tricuspid valve", section on 'Tricuspid stenosis'.)

●Doppler echocardiography reveals high velocity turbulent diastolic flow across the stenotic orifice and prolonged pressure half-time (T_1/2).

•With severe TS, the pressure half-time is ≥190 ms and the valve area is ≤1 cm². The tricuspid valve area in cm² may be estimated by dividing 190 by the pressure half-time [33].

•With severe TS, the gradient across the tricuspid valve is variable, as it is affected by heart rate, forward flow, and respiratory phase.

-With preserved cardiac output and a heart rate of 70 bpm, the mean pressure gradient is usually 5 to 10 mmHg. Higher gradients may occur with combined TS and tricuspid regurgitation [34].

-With a low output state (such as with concomitant severe mitral stenosis), severe TS is associated with lower tricuspid valve gradients. When the gradient is low, other echocardiographic features may suggest severe TS.

●The hemodynamic effects of TS cause dilation of the right atrium and inferior vena cava. (See "Echocardiographic evaluation of the atria and appendages", section on 'Right atrium' and "Echocardiographic assessment of the right heart", section on 'RA pressure'.)

Echocardiographic findings may also help establish the etiology of TS. For example, presence of pacemaker leads, particularly looped leads, suggests pacemaker lead-related TS.

It is also important to assess the presence and severity of concomitant tricuspid regurgitation since this can influence the decision to proceed with tricuspid valve surgery or balloon valvotomy. In patients with concomitant tricuspid regurgitation, the higher flow through the tricuspid valve further increases the gradient across the valve and the elevation of right atrial pressures [7]. (See 'Indications for intervention' below and "Echocardiographic evaluation of the tricuspid valve".)

The presence of concomitant valve disease should also be assessed, particularly in patients with rheumatic heart disease who commonly have mitral valve disease and sometimes also aortic valve disease.

Three-dimensional transesophageal echocardiography provides better qualitative data on the morphological abnormalities seen on two-dimensional echo as it allows for en face visualization of the valve, and planimetry of the valve area can be performed. However, the utility of three-dimensional transesophageal echo for assessment of TS has not been systematically studied.

Cardiac catheterization — Right heart catheterization is performed with two catheters or one double lumen catheter. After calibration of the two transducers, it is important first to document identical simultaneous recordings of right atrial pressure and then to advance one of the catheters into the right ventricular cavity. Simultaneous recordings of atrial and ventricular pressure are made in at least 8 to 10 cardiac cycles.

As noted above, at a heart rate of 70 bpm, the mean pressure gradient in severe TS is usually 5 to 10 mmHg. Diastolic pressure gradients as low as 2 to 4 mmHg may indicate severe TS; gradients rarely exceed 10 mmHg. Thus, respiratory variation of a relatively low pressure gradient or non-simultaneous measurements may provide misleading data. A single catheter "pull-back" from the ventricle to the atrium does NOT provide a sufficiently accurate measurement of the pressure gradient. (See "Hemodynamics of valvular disorders as measured by cardiac catheterization".)

Cardiac output is measured using the Fick principle, and the valve area is calculated in a manner similar to that used to calculate the mitral valve area. A tricuspid valve area less than 1 cm² indicates severe TS [35]. (See "Hemodynamics of valvular disorders as measured by cardiac catheterization", section on 'Tricuspid stenosis'.)

Differential diagnosis — The differential diagnosis of TS includes other causes of systemic venous hypertension and right heart failure, such as tricuspid regurgitation, right ventricular dysfunction, constrictive pericarditis, and restrictive cardiomyopathy [36]. The conditions are generally distinguished from TS by echocardiography.

TS has characteristic tricuspid valve morphological abnormalities and Doppler findings. These tricuspid abnormalities occur in the absence of ventricular dysfunction, other valve disease, or pericardial disease. (See "Physiologic and pharmacologic maneuvers in the differential diagnosis of heart murmurs and sounds".)

STAGING — Severe TS is identified by valve anatomy, valve hemodynamics, and hemodynamic consequences largely based upon echocardiographic findings as described above [32]. Symptoms of severe TS vary from none to severe, depending upon degree of obstruction and coexisting valve disease. (See 'Echocardiography' above and "Echocardiographic evaluation of the tricuspid valve", section on 'Tricuspid stenosis'.)

No criteria for Stages A or B were included in the 2014 American Heart Association/American College of Cardiology valve guidelines [32]. Stage C is defined as severe TS without symptoms. Stage D is defined as severe TS with symptoms.

TREATMENT

Approach to treatment — Limited data are available to guide treatment of TS. Since TS is a mechanical disorder, it is most effectively treated by valve intervention with either valve surgery (valve repair or replacement) or percutaneous balloon valvotomy. Medical management with diuretic therapy may help reduce symptoms of congestion and thus may be helpful prior to intervention as well as in patients who are not candidates for intervention.

Thus, treatment of many patients with TS involves a combination of medical therapy and valve intervention. As an example, treatment of symptomatic severe TS related to pacemaker leads generally includes removal of the involved endocardial lead(s) (which may be replaced by an epicardial system for patients who continue to require a pacemaker), tricuspid valve surgery, and treatment of any associated infection. Case reports have described percutaneous valvotomy as a potential alternative to surgery in selected patients with lead-associated TS [37-39].

Medical management — In patients with severe TS, symptoms of systemic venous hypertension, including lower extremity edema and hepatic congestion, are treated with loop diuretics [32,40]. However, diuretic therapy has limited efficacy in patients with TS and use may be limited by worsening low-flow syndrome. (See "Right heart failure: Causes and management", section on 'Management'.)

Specific therapy is indicated for treatment of some of the causes of TS, such as systemic rheumatic diseases and endocarditis. In some instances, treatment of the underlying etiology (systemic lupus erythematosus and antiphospholipid syndrome) may reduce the degree of stenosis [41]. Reversal of tricuspid valve abnormality has also been documented with the cessation of fenfluramine or methysergide use [42]. (See "Atrial fibrillation: Overview and management of new-onset atrial fibrillation" and "Rheumatic mitral stenosis: Overview of management" and 'Etiology' above and "Valvular heart disease induced by drugs".)

Medical therapy is also indicated for associated conditions such as atrial fibrillation. (See "Atrial fibrillation: Overview and management of new-onset atrial fibrillation".)

Data on outcomes of medical management of TS are limited, and controlled data are lacking. A retrospective series indicated that some patients with significant TS and systemic venous hypertension have prolonged survival [5], but limited information is available on the risk of morbidity and mortality in such patients. Of 13 patients with severe TS included in the study, 12 underwent mitral and/or aortic valve surgery; six underwent concomitant tricuspid valve repair and six underwent no intervention. At 6- to 31-year follow-up, 9 of the 13 patients were in New York Heart Association functional class I or II; the medical treatment received by these nine patients was not specified.

Intervention

Indications for intervention — Tricuspid valve intervention for TS (ie, valve surgery [valve repair or replacement] or percutaneous balloon valvotomy) is generally reserved for symptomatic patients with severe TS. Among patients with symptomatic severe TS who are candidates for intervention, a choice is made between valve surgery and balloon valvotomy based upon considerations including surgical risk, the presence of tricuspid regurgitation and risk of worsening regurgitation with valvotomy, and operator experience.

●For patients with isolated symptomatic severe TS with low to moderate surgical risk, we suggest tricuspid valve surgery rather than balloon valvotomy. Tricuspid valve surgery (valve repair or replacement) is generally preferred over percutaneous balloon tricuspid valvotomy in this setting since there is greater experience with surgery, most cases of severe TS are accompanied by tricuspid regurgitation, and balloon valvotomy may create or worsen tricuspid regurgitation [32].

●For patients with isolated symptomatic severe TS and mild or less tricuspid regurgitation with high surgical risk, we suggest balloon valvotomy. Tumor masses, vegetations, and thrombi are contraindications to valvotomy.

●For patients with isolated symptomatic severe TS and moderate or greater tricuspid regurgitation with high surgical risk, we perform an individualized assessment of the risks and benefits of tricuspid valve surgery versus medical therapy.

●For patients with severe TS requiring surgery for left-sided valve disease, we suggest concomitant tricuspid valve surgery.

Similar recommendations are included in the 2014 with 2017 update American Heart Association/American College of Cardiology (AHA/ACC) valve guideline and in the 2017 European Society of Cardiology (ESC) valve guidelines [32,43,44].

Limited evidence is available to guide management of TS, so recommendations for treatment of TS are largely based upon expert opinion and limited observational data. Few data are available on outcomes in patients with TS with medical management intervention (tricuspid valve surgery or percutaneous valvotomy). Studies comparing percutaneous balloon valvotomy with tricuspid valve surgery are not available. Reports of tricuspid balloon valvotomy are scant [45,46] and lack long-term follow-up. Larger studies reporting surgical outcomes for tricuspid valve surgery did not separate cases with predominant TS from those with predominant tricuspid regurgitation [47,48]. (See 'For valve surgery' below.)

Management of tricuspid prosthetic valve obstruction is discussed separately. (See "Bioprosthetic valve thrombosis, thromboembolism, and obstruction: Clinical manifestations and diagnosis" and "Bioprosthetic valve thrombosis, thromboembolism, and obstruction: Management" and "Mechanical prosthetic valve thrombosis or obstruction: Clinical manifestations and diagnosis" and "Management of mechanical prosthetic valve thrombosis and obstruction".)

Outcomes and complications — Outcomes following intervention in patients with severe TS have not been well defined. Isolated TS is uncommon, so most patients undergoing intervention for TS undergo concomitant intervention for concurrent mitral and/or aortic disease.

For valve surgery — Limited data are available on efficacy and outcomes of tricuspid valve surgery for patients with TS. In surgical series, patients with predominant TS are often combined with those with predominant tricuspid regurgitation or mixed stenosis and regurgitation and other types of valve disease [21,32,47,48].

Complications following tricuspid valve surgery are the same as those for other valve surgery, including mortality, prolonged ventilation, renal failure, pneumonia, sepsis, neurologic complications, bleeding, and multiorgan failure. The atrioventricular node may be injured when suturing in the region of the anteroseptal commissure encroaches upon myocardial tissue at the atrioventricular node [21]. A minority of patients undergoing tricuspid valve surgery develop atrioventricular block, requiring permanent pacemaker placement. The rate of pacemaker placement is higher following tricuspid valve replacement than following tricuspid valve repair (eg, 17.2 versus 9.5 percent) [48]. Iatrogenic stenosis of the right coronary artery has been reported and may be caused by tension on adjacent tissues causing functional stenosis or by direct suturing of the artery [21]. (See "Postoperative complications among patients undergoing cardiac surgery".)

Operative mortality for tricuspid valve surgery is approximately 10 percent. A Society of Thoracic Surgeons (STS) database study reported an operative mortality for tricuspid valve surgery (repair or replacement) of 10 percent, which remained similar from 1993 to 1997 and 2003 to 2007 [47]. Similarly, a study of isolated valve surgery outcomes using the Nationwide Inpatient Sample (NIS) database from 1999 to 2008 reported an overall hospital mortality of 10.6 percent [48]. The presence of liver disease was a risk factor for mortality.

Most tricuspid valve surgery (eg, 80 percent [48]) is performed as a concomitant procedure with other cardiac surgery, and surgery involving more than one valve is generally associated with an increased risk compared with isolated tricuspid valve surgery. In the NIS database study, hospital mortality for tricuspid surgery plus other cardiac surgery was 16.1 percent (significantly higher than for tricuspid surgery alone at 10.6 percent) [48]. In the STS database study, operative mortality for mitral plus tricuspid valve surgery was 8.7 percent during 2003 to 2007, operative mortality for aortic plus tricuspid valve surgery was 11.8 percent during 2003 to 2007, and operative mortality for combined aortic, mitral, and tricuspid valve surgery was 13.1 percent [47].

Few studies have reported long-term survival after tricuspid valve replacement (for tricuspid regurgitation or TS) and data for controls are generally not available:

●A review reported survival rates for hospital-discharged patients with tricuspid valve prostheses with mean age 49.3 at implantation [49]. Survival rates at 1, 5, 10, and 15 years were 86.9, 73.5, 60.2, and 47.8 percent for patients with mechanical tricuspid prostheses and 86.7, 73.6, 62.0, and 46.7 percent for patients with bioprosthetic tricuspid valves.

●Long-term follow-up was reported for 530 patients undergoing cardiac surgery that included a tricuspid valve procedure (66 percent repair and 34 percent replacement) at a mean age of 56.9 years. Of the patients undergoing tricuspid valve replacement, 86.6 percent received a bioprosthetic valve. Actuarial survival rate was 20 percent at 15 years.

For balloon valvotomy — Data on outcomes following balloon valvotomy for TS are limited. There is far less experience with tricuspid valvotomy than with mitral valvotomy. Valve areas generally increase from less than 1 to almost 2 cm² [50]. While some stenosis persists, this change in area is sufficient to produce a significant reduction in the transvalvular pressure gradient and a decrease in right atrial pressure. Successful valvotomy using one, two, or three balloons has been described [51-53]. Similar beneficial results have been described when balloon valvotomy is used to treat combined TS and mitral stenosis, as well as in cases of combined tricuspid, aortic, and mitral stenosis [45,53,54].

Complications for tricuspid balloon valvotomy include new or worsened tricuspid regurgitation (generally mild in published reports), bleeding, and access site complications.

Limited data are available on long-term outcomes. A report of four patients with severe TS who were successfully treated with balloon valvotomy found that symptomatic and hemodynamic improvements persisted at three-year follow-up [55].

Surgical valve replacement or repair — Among patients with TS undergoing tricuspid valve surgery, most patients require tricuspid valve replacement given the extent of valvular and subvalvular disease. Surgical tricuspid valve repair is attempted if feasible (eg, with just one or two fused commissures), but valve replacement is performed if repair is not adequate.

Limited data are available comparing surgical tricuspid repair and surgical tricuspid valve replacement in patients with TS:

●Hospital mortality is higher in patients undergoing tricuspid valve replacement compared with those undergoing repair (for tricuspid regurgitation and/or TS), but at least part of this difference may be due to differences in risk factors. In a study of 28,726 admissions for tricuspid valve surgery during 1999 to 2008, hospital mortality for tricuspid valve replacement was higher than for tricuspid valve repair (13.6 versus 9.5 percent) [48]. However, the difference in mortality between the replacement and repair groups was no longer significant in the subset of patients with liver disease undergoing tricuspid valve surgery. Mortality for replacement remained higher than for repair in the subset of patients without liver disease. Further risk adjustment was not performed.

●Long-term mortality rates may be similar. A report of a series of 530 patients undergoing tricuspid valve surgery (for tricuspid regurgitation and/or TS) from 1961 to 1987 found no difference in survival rates or reoperation rates following tricuspid valve repair versus replacement at median four-year follow-up [56].

Choice of prosthesis in patients requiring valve replacement — For patients requiring tricuspid valve replacement for TS, a bioprosthetic or mechanical valve may be chosen, and evidence to guide valve choice is limited. Considerations are similar to those for choice of a prosthetic valve generally (based largely on mitral and aortic data), but data specific to tricuspid valve replacement are limited [57]. As for prosthetic valves in any position, for patients with limited life expectancy, the increased ongoing risk of anticoagulation to avoid thrombotic complications with a mechanical valve may outweigh the long-term risk of valve failure due to structural valve degeneration with a bioprosthetic valve. (See "Choice of prosthetic heart valve for surgical aortic or mitral valve replacement".)

Available data suggest no survival difference based upon prosthetic type for tricuspid valve replacement (combining TS and tricuspid regurgitation cases). A systematic review reported a pooled survival hazard ratio for mechanical versus bioprosthetic tricuspid valve of 1.07 (95% CI 0.84-1.35) [49]. The pooled freedom from reoperation hazard ratio was 1.24 (95% CI 0.64-2.31).

For valve replacement for TS, the 2014 AHA/ACC valve guideline noted only that the choice of prosthesis should be individualized [32]. The 2012 ESC valve guidelines indicate that a bioprosthesis is usually preferred over a mechanical prosthesis in this setting due to concern of higher risk of thrombosis with mechanical valves and satisfactory durability of tricuspid bioprosthetic valves but acknowledge that this is still a matter of debate [40].

PREGNANCY — Management of pregnancy in patients with TS is discussed separately. (See "Pregnancy and valve disease", section on 'Tricuspid stenosis'.)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Cardiac valve disease".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, “The Basics” and “Beyond the Basics.” The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on “patient education” and the keyword[s] of interest.)

●Basics topics (see "Patient education: Tricuspid stenosis (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Tricuspid stenosis (TS) is most commonly of rheumatic etiology. Rheumatic TS almost never occurs as an isolated lesion; it is often accompanied by tricuspid regurgitation and is generally associated with mitral valve disease and, in some cases, aortic valve disease. (See 'Etiology' above.)

●In patients with TS, obstruction to tricuspid flow limits cardiac output, causes fatigue, and produces symptoms and signs of systemic venous hypertension. Concomitant mitral or aortic disease may cause additional symptoms such as dyspnea. (See 'Clinical manifestations' above.)

●TS should be suspected in patients with signs and symptoms of systemic venous hypertension, particularly those with conditions associated with TS. TS is generally diagnosed and evaluated by transthoracic echocardiogram. (See 'Approach to diagnosis and evaluation' above.)

●Echocardiography is the most important modality utilized to identify TS. Severe TS is identified by the characteristic abnormal morphology on two-dimensional imaging accompanied by Doppler identification of a pressure half-time ≥190 ms and valve area ≤1 cm². Stage C is defined as severe TS without symptoms. Stage D is defined as severe TS with symptoms. (See 'Staging' above.)

●Since TS is a mechanical disorder, it is most effectively treated by valve intervention, ie, valve surgery (valve repair or replacement) or percutaneous balloon valvotomy. Medical management with diuretic therapy may help reduce symptoms of congestion and thus may be helpful prior to intervention as well as in patients who are not candidates for intervention. (See 'Approach to treatment' above.)

●Tricuspid valve intervention for TS (ie, valve surgery [valve repair or replacement] or percutaneous balloon valvotomy) is generally reserved for symptomatic patients with severe TS. (See 'Indications for intervention' above.)

•For patients with isolated symptomatic severe TS with low to moderate surgical risk, we suggest tricuspid valve surgery rather than balloon valvotomy (Grade 2C).

•For patients with isolated symptomatic severe TS and mild or less tricuspid regurgitation with high surgical risk, we suggest balloon valvotomy (Grade 2C). Tumor masses, vegetations, and thrombi are contraindications to valvotomy.

•For patients with isolated symptomatic severe TS and moderate or greater tricuspid regurgitation with high surgical risk, we perform an individualized assessment of the risks and benefits of tricuspid valve surgery versus medical therapy.

•For patients with severe TS requiring surgery for left-sided valve disease, we suggest concomitant tricuspid valve surgery (Grade 2C). (See 'Indications for intervention' above.)

●Patients undergoing tricuspid valve surgery for TS generally require valve replacement, with surgical tricuspid valve repair attempted only when deemed feasible (eg, with just one or two fused commissures). (See 'Surgical valve replacement or repair' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges William H Gaasch, MD (deceased), who contributed to earlier versions of this topic review.